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Primary Mucosa-Associated Lymphoid Tissue Lymphoma of the Rectum


1 Department of Radiation Oncology, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, IR Iran
2 Colorectal Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran
3 Dr Daneshbod pathology laboratory, Shiraz, IR Iran
4 Shiraz Institute for Cancer Research, Shiraz University of Medical Sciences, Shiraz, IR Iran
*Corresponding author: Hamid Nasrolahi, Department of Radiation Oncology, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, IR Iran. Tel: +98-7116125170, Fax: +98-7116474320, E-mail: nasrolahihamid@yahoo.com.
Annals of Colorectal Research. 1(3): 106-110. , DOI: 10.17795/acr-13953
Article Type: Case Report; Received: Jul 31, 2013; Revised: Sep 12, 2013; Accepted: Nov 18, 2013; epub: Dec 30, 2013;

Abstract


Introduction: Mucosa-associated lymphoid tissue (MALT) lymphoma most commonly involves the stomach and its treatment is surgery, antibiotic therapy or radiotherapy. Rectal MALT is not only so rare but treatment is not as clear as gastric MALT.

Case Presentation: Our patient was a 66 year-old man presenting with 5-6 months abdominal pain and anemia. In colonoscopy, a rectal ulcer was found and biopsy revealed MALT lymphoma. Physical examination and staging evaluations were normal. Radiotherapy was started for him but he did not tolerate radiotherapy and instead received chemotherapy. After 38 months, he is fine.

Conclusions: The incidence of rectal MALT is rare and treatment is not so clear. The results on H. pylori eradication, radiotherapy and chemotherapy are more controversial.

Keywords: Rectum; Lymphoma, B-Cell, Marginal Zone; Drug Therapy; Radiotherapy

1. Introduction


Lymphoma is the 6th common cause of cancer related death in America and unfortunately its incidence is increasing (1). Among lymphomas Mucosa-Associated Lymphoid Tissue (MALT) lymphoma consists of 5% of them. The most frequently involved organ by MALT is the stomach (2). The first recommended treatment in gastric MALT lymphoma is H. pylori eradication. Other therapeutic modalities are also effectively used. Radiotherapy is administered if antibiotic therapy is not successful. Systemic agents such as rituximab and chemotherapy are reserved for disseminated patients (3). Although gastrointestinal (GI) tract is the most location for extra nodal lymphoma, colorectal lymphoma is rare and even rectal primaries are less common (1). For rectal MALT lymphoma, treatment is not as clear as in gastric cases. We here present a man with rectal MALT lymphoma and his treatment course.

2. Case Presentation


The patient was a 66 year-old man with abdominal pain for 5-6 months. Physical examination was normal with no significant positive past medical or family history. Abdominal sonography and blood chemistry tests were normal. Complete blood count showed anemia (Hb = 10). Upper endoscopy was done for him, revealing no pathology and urease test for H. pylori was negative. Colonoscopy was performed. In the rectum, a large single ulcer was found and biopsy showed MALT lymphoma. Immunohistochemistry was performed, being positive for CD20 and Bcl-2 which is consistent with low grade B-cell lymphoma (Figures 1,2,3 and 4). In May 2010, the patient was referred to our radiation oncology ward for treatment. Physical examination, blood chemistry tests and LDH were normal. Staging work up including the chest, abdomen and pelvic CT scan showed no evidence of tumor spread. We started radiotherapy to the whole pelvis with 1.8 Gy per fraction for him but during the treatment he developed severe abdominal pain and fresh rectal bleeding. Supportive care was not effective and radiation was stopped after 2200 cGy. After one month, the patient’s has improved and radiotherapy was restarted but rectal bleeding was restarted after one day. So irradiation was discontinued and chemotherapy was started for him. He received six cycles of CHOP (cyclophosphamide, vincristine, doxorubicin and prednisolone) and 38 months after the diagnosis, he is completely fine and is under regular follow up.

Figure 1.
Section of Rectal Mucosal Lesion Shows Diffuse Small to Medium Sized Lymphocytic Infiltration With Dispersed Chromatin and Inconspicious Nucleoli, and Pale Cytoplasm With Crypt Detruction. Focal Plasmacytic Differentiation Is Present (x200, H&E).
Figure 2.
Lymphocytes Show Diffuse CD20 Positivity (Immunoperoxidase Stain).
Figure 3.
Lymphocytes Are Diffuse Bcl-2 Positive (Immunoperoxidase Stain).
Figure 4.
Lymocytes Are Mainly Diffuse CD3 Negative With Few Scattered Positive Cells (Immunoperoxidase Stain).

3. Discussion


Gastrointestinal tract is the most location in primary extranodal lymphoma but colorectal lymphoma is not so common. Colorectal lymphomas are only 1.4% of Non Hodgkin’s Lymphoma (NHL) and less than 1% of colorectal malignancies. In 70% of colorectal lymphomas, the cecum and ascending colon are involved. Histologically, diffuse large B-cell lymphoma is the most common histologic type. MALT is less common than LBCL and is an indolent disease ( 1 ). Ahlawat in 2006 found 30 reports on rectal MALT in English literature and the majority were Japanese ( 4 ). We summarized the cases found in Pubmed with key words ”Lymphoma” ,“Rectal”, “Rectum” and “MALT” in Table 1. We found 25 cases in English literature (Table 1). We had no access to clinical data of 10 patients in three reports ( 5 - 7 ).

Table 1.
Reports and Results on Rectal Lymphoma

Because of the rarity of this kind and location of the tumor, there is no consensus on treatment. Some authors suggest no treatment more than resection for colorectal MALT (1). Orita et al. reported a case with rectal MALT who had not received any treatment after tumor resection. After 35 months, the tumor was well controlled (20). Even spontaneous tumor regression has been reported. Takenaka et al. reported an old man who was not operable and because of multiple underlying diseases he did not receive chemotherapy or radiotherapy. He was a 76 year-old man with renal and heart disease. His disease showed spontaneous regression and after 19 months he was tumor free (21).

It is well known that H. pylori eradication is an effective treatment for gastric MALT (2). But regarding rectal MALT, the results are controversial. Grunberger et al. in a prospective study evaluated the role of antibiotic therapy for H. pylori positive extragastric MALT. Among 16 patients who were H. pylori positive and were treated for eradication, only one case showed tumor response. Others showed no response and received local therapy (8). Amouri et al. reported a 46 year-old woman with rectal MALT. Because she had H Pylori infection, first she received antibiotic therapy for Hpylori eradication. But her disease had no improvement, then she received 34 Gy with complete response (22). Nakase et al. reported tumor regression after antibiotic therapy for H. pylori in three H.pylori negative rectal MALT patients (23). There are other reports on successful MALT treatment with antibiotics in H.pylori negative patients (9, 24). They suggested that micro-organisms that are different from H. pylori are responsible for rectal MALT.

Gastric MALT is radiosensitive and usually well controlled by 30 Gy radiation (10). Regarding radiotherapy, although few reports are available, the results are more similar to those of gastric MALT. Isobe and Tsang showed moderate dose radiotherapy is effective in achieving durable response in MALT (25, 26). Goda et al. showed similar results. In their study among 167 patients, 31 recurrences occurred and seven cases had transformation to DLBCL (27). Studies mentioned included MALT lymphoma of different organs, mostly the orbit, thyroid, salivary glands and stomach. Due to its rarity, rectal MALT has not been studied widely. Foo et al. reported a 60 year-old man with rectal MALT who was treated with 45 Gy and had complete response (11). Kobayashi et al. reported a 26 year-old man who underwent only biopsy of the rectum and received adjuvant radiotherapy with complete response (12). We first decided to prescribe 30 Gy to the pelvis, but unfortunately he developed severe dysuria and fresh rectal bleeding. So, radiotherapy was stopped after 24 Gy and chemotherapy was started for him.

Our patient may be considered as a case of overtreatment. Although radiation with fractionated doses as low as 25 Gy has been used for nodal MALT lymphoma, most reports series on Rectal lymphoma as listed in the Table 1, used at least 30 Gy ( 28 ). Our patient did not complete his planned radiation up to 30 Gy; and due to persistent rectal bleeding, chemotherapy was recommended for tumor control.

Although most MALT lymphomas are well controlled by local therapy (radiotherapy or surgery), there are reports in which MALT has a higher rate of systemic recurrence than previously assumed. Raderer et al. found that one third of gastric lymphoma and half of the extragastric MALT lymphomas are systemic (29). Zinzani et al. used chemotherapy in 31 non-gastric MALTs. Eleven cases received CVP (cyclophosphamide, vincristine and prednisolone) and 20 cases FM (fludarabine-mitoxantrone) and all had complete response. Four cases developed recurrence after receiving CVP and received FM as the second line which was successful. Five year survival was 100% (30). Rituximab is a CD20 antibody and is successfully used for MALT in other organs (3). Kagawa administered this agent in a case of rectal MALT with complete remission (13).

Ersoz et al. reported a 44 year-old woman who had no evidence of H. pylori infection. After complete tumor removal, she received CHOP as chemotherapy. They believed that local tumor removal and chemotherapy is the best treatment for rectal MALT (2). Because our patient could not complete radiotherapy course, we prescribed six cycles of CHOP. Controversy on rectal MALT is continuing and more studies including more patients with more follow up duration are needed to make a more reliable conclusion.

Acknowledgments

The authors would like to thank Dr. Nasrin Shokrpour at Center for Development of Clinical Research of Nemazee Hospital for editorial assistance.

Footnotes

Implication for health policy/practice/research/medical education: This article reports a man with rectal MALT lymphoma. It is not only a rare disease, but also no definite treatment is known. We herein report this case and review literature for similar reports and outcome.
Authors’ Contribution: Shapour Omidvari: involved in conception, data collection, literature review, writing the manuscript and approval of final version. Hamid Nasrolahi: involved in conception, data collection, literature review, writing the manuscript and approval of final version and to accept responsibility for contents of final manuscript. Sayed Hasan Hamedi: involved in conception, data collection, writing the manuscript and approval of final version. Mohammad Mohammadianpanah: Involved in design and in data collection, and interpretation, and in literature review, writing, and revising the manuscript, and approval of final version. Yahya Daneshbod: Involved in conception, design, data collection, literature review and in writing the manuscript and approval of final version. Ahmad Mosalaei: involved in conception, data collection, writing the manuscript and approval of final version. Niloofar Ahmadloo: Involved in design and in data collection, analysis, and interpretation, and in writing, and revising the manuscript, and approval of final version. Mansour Ansari: involved in data collection, literature review, writing the manuscript and approval of final version.
Financial Disclosure: No financial disclosure.
Funding/Support: No fund or support.

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Table 1.

Reports and Results on Rectal Lymphoma

AuthorAge, yGenderSurgeryChemotherapyRadiotherapy, GyAntibiotic Therapy DFS a, mo
Ersoz (2)42femaletrans-anal resectionCHOP anono
Amouri (8)46femalenono34yes
Nomura (9)53femalenononoyes
Okamura (10), 3 cases
56femalenono30no75
62femalenono30no13
65malenono30no65
Foo (11)60malenono45no41
Kobayashi (12)26malenonoyesno
Kagawa (13)62malenorituximabnono
Park (14)44malenoCHOPnono11
De Sanctis (15)66malenononoyes34
Yamamoto (16)33femalenono30yes
Watanabe (17)75femaleresection 3 timesnonono48
Chahil (18)78femalenonoyesyes
Samee (19)68femaleyesnoyesno48
Present case66malenoCHOP 24no38
a Abbreviation: CHOP, Cyclophosphamide; DFS, Disease Free Survival

Figure 1.

Section of Rectal Mucosal Lesion Shows Diffuse Small to Medium Sized Lymphocytic Infiltration With Dispersed Chromatin and Inconspicious Nucleoli, and Pale Cytoplasm With Crypt Detruction. Focal Plasmacytic Differentiation Is Present (x200, H&E).

Figure 2.

Lymphocytes Show Diffuse CD20 Positivity (Immunoperoxidase Stain).

Figure 3.

Lymphocytes Are Diffuse Bcl-2 Positive (Immunoperoxidase Stain).

Figure 4.

Lymocytes Are Mainly Diffuse CD3 Negative With Few Scattered Positive Cells (Immunoperoxidase Stain).